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management rheumatology dermatomyositis activation antimda5 antimda5dm antinxp2 antinxp2dm common hemophagocytic hlh hypogammaglobulinemia immunodeficiency immunology lymphohistiocytosis macrophage mas treatment variable
Common Variable Immunodeficiency (CVID) What? CVID: Immunodeficiency disorder with hypogammaglobulinemia -> increased infection risk secondary to impaired B-cell
to 8 years Clinical ... • Chronic lung ... : • Nephrotic syndrome ... (Epstein-Barr syndrome ... hypogammaglobulinemia #immunology #hematology
Macrophage Activation Syndrome (MAS) Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH) What? A subset of
Clinical Presentation ... rash) • Heart, lung ... 5000 to 10,000 ng/mL ... #Diagnosis #Management ... #Hematology #Rheumatology
Hemophagocytic Lymphohistiocytosis (HLH) Definition: Overwhelming clinical syndrome associated with excessive macrophage activation and cytokine storm due to
: Overwhelming clinical ... syndrome associated ... : HLH signs and ... Ferritin >500 ng/mL ... management #treatment #hematology
ANTI-MDA5 DERMATOMYOSITIS Cutaneous manifestations: • Periorbital heliotrope (blue-purple) rash with edema • Erythematous rash on the face, or the
chest (in a V-sign ... dorsal part of the MCP ... auricular papules Clinical ... anti-NXP2 Abs Lung ... #dermatology #diagnosis
Anti-NXP-2 Dermatomyositis What? DM with generalized subcutaneous edema Pts have more myalgias, more severe weakness, and an increased prevalence
chest (in a V-sign ... dorsal part of the MCP ... auricular papules Clinical ... Non-small-cell lung ... #dermatology
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